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Acute annular urticaria is a common and benign cutaneous hypersensitivity reaction seen in children that manifests with characteristic annular, arcuate, and polycyclic urticarial lesions in association with acral edema. It is mistaken most often for erythema multiforme and, occasionally, for a serum-sickness–like reaction. Although these 3 entities may present in a similar manner, specific clinical features help to distinguish them, and it is important for the clinician to be able to differentiate among them. We present herein a series of 18 patients who were given a diagnosis of acute annular urticaria and review the clinical distinctions between acute annular urticaria, serum-sickness–like reactions, and erythema multiforme. Because of the frequency of its clinical confusion with erythema multiforme, we propose the term “urticaria multiforme” as a more apt description to highlight the distinctive clinical features of this urticaria variant.

Purpose of review Cutaneous fungal infections are not uncommon in newborns and are seen in premature or otherwise immunocompromised neonates as well as in healthy full-term neonates. Healthy newborns can develop clinical manifestations as a result of infection with Candida species or as a result of skin colonization with Malassezia species; cutaneous infection with other fungal pathogens is rare. Immunocompromised and premature neonates, however, are susceptible to infection with opportunistic pathogens and are also at higher risk for invasive infection with common pathogens such as Candida. This review discusses the fungal species associated with cutaneous fungal infection in neonates, emphasizes the relevant clinical features, and also reviews the use of newer antifungal agents, including lipid-associated amphotericin B, voriconazole, and caspofungin. Recent findings Neonatal cutaneous infections with opportunistic fungal pathogens, including Aspergillus and the Zygomycetes, have been reported with increasing frequency as advances in neonatal care have improved the survival rate in very low birthweight neonates. Although these infections are frequently fatal, survival in some neonates has been reported with the use of aggressive surgical debridement and systemic antifungal therapy. Newer antifungal agents, including voriconazole and caspofungin, show promise in the treatment of potentially fatal fungal infections in neonates. Summary Cutaneous fungal infections in neonates range from generally benign conditions such as congenital candidiasis and neonatal cephalic pustulosis to potentially fatal infections with opportunistic pathogens in very low birthweight or immunocompromised neonates. The prompt recognition and appropriate treatment of cutaneous fungal disease in neonates is critical to the prevention of adverse outcomes.

Objectives. Terbinafine has been shown to be effective in tinea capitis, using different treatment durations. However, no direct comparison of treatment duration has previously been investigated. This randomized, double-blind, parallel-group, multicenter study was designed to assess the effect of terbinafine treatment duration on the outcome of Trichophyton tinea capitis in a North American population. Methods. A total of 176 patients with a clinical diagnosis of tinea capitis were enrolled in this study and treated with oral terbinafine (3–6 mg/kg/d) for 1, 2, or 4 weeks. All patients were to be followed until week 12. A total of 159 patients had culture-confirmed tinea capitis attributable to Trichophyton species and constituted the intent-to-treat population used for efficacy analysis (50, 55, and 54 patients in the 1-, 2-, and 4-week arms, respectively). Results. At the end of study, effective treatment, defined as negative culture and low scores on signs and symptoms, was achieved in 56%, 69%, and 65% of patients who were treated with terbinafine for 1, 2, and 4 weeks, respectively. A negative culture was achieved in 60%, 76%, and 72%, respectively. Overall, the efficacy data showed that both the 2- and 4-week treatment regimens are clinically superior to the 1-week regimen. Terbinafine was well tolerated, and the incidence of adverse events showed no relationship to the duration of therapy. Conclusion. When efficacy, cost, and compliance are taken into consideration, 2 weeks of terbinafine therapy appears to be the optimal treatment duration for patients with Trichophyton tonsurans tinea capitis.

Aquagenic palmoplantar keratoderma is an acquired condition characterized by burning and edema limited to the hands after brief immersion in water. The 3 patients described possess a striking similarity to those with transient reactive papulotranslucent acrokeratoderma. All 3 patients manifested the "hand-in-the-bucket" sign, having presented to a physician with a hand immersed in a bucket of water to more promptly demonstrate the physical findings. Aluminum chloride hexahydrate represents a potentially valuable therapeutic option for this unusual condition.

A case of forehead lipoblastoma simulating a hemangioma in a male infant is reported, to alert pediatricians to this rare tumor and to increase the index of suspicion in atypical hemangiomas. A 2-month-old male infant developed a protruding forehead mass with increased vascularity. It demonstrated progressive and accelerated growth over the subsequent 6 months, unresponsive to steroid therapy. A magnetic resonance imaging scan supported the diagnosis of hemangioma because of the hypervascular nature of the lesion. Surgical excision was performed because of visual obstruction. Pathologic examination of the specimen was consistent with a very primitive lipoblastoma. This tumor is a rare, benign lesion of immature fat cells that is found almost exclusively in the pediatric population. Lipoblastomas are more common in males than females and frequently present as asymptomatic, rapidly enlarging, soft lobular masses on the extremities. Complete surgical excision is the definitive treatment. In the vast majority of reported cases, however, the preoperative diagnosis was incorrect, underscoring the diagnostic dilemma presented by these rare tumors.

Objective. Blue light phototherapy is commonly administered to neonates as treatment of indirect hyperbilirubinemia, often in conjunction with blood transfusions to treat hemolytic anemia. We observed a distinctive cutaneous complication of phototherapy in six neonates with hyperbilirubinemia. Methodology. We studied the clinical and histologic characteristics of the eruption, as well as the porphyrin levels in affected neonates. Five of the patients had erythroblastosis fetalis; the other had profound anemia from twin–twin transfusion. All of the neonates developed purpuric patches at sites of maximal exposure to the phototherapy lights, with dramatic sparing at shielded sites within 24 hours after initiation of the phototherapy. On discontinuation of phototherapy, all eruptions cleared within 1 week. Examination of skin biopsy sections showed purpura without significant inflammation or keratinocyte necrosis. Plasma porphyrins (copro- and proto-) were elevated in the two patients in which they were assessed. Conclusions. The distribution of the eruption in areas exposed to light and presence of circulating porphyrins suggest that porphyrinemia may underlie the light-induced purpuric eruption. Additional studies will be required to determine definitively the mechanisms of both the purpuric phototherapy-induced eruption and the development of increased blood porphyrin levels in these transfused neonates.

We observed a newborn infant of a previously reported kindred with absent dermal ridge pattern, syndactyly, and facial milia. The infant's features were consistent with three other kindreds, suggesting that this entity is a single disorder with variable expression. Furthermore, this entity should be considered in the differential diagnosis of excessive congenital facial milia and erosions.

Introduction Atopic dermatitis, also referred to as atopic eczema, infantile eczema, allergic eczema, disseminated neurodermatitis, and prurigo Besnier, is a common and important cause of morbidity in children of all ages. A total of 22% of patients seen in pediatric dermatology clinics have atopic dermatitis. In 1969, Wingert et al reported that 4% of pediatric emergency room visits at the Los Angeles County General Hospital were due to atopic dermatitis, and this did not include patients seen for impetigo, a common complication of atopic dermatitis. The prevalence of atopic dermatitis in the pediatric population has increased over the past 3 decades from 3% to 10%, and it appears to be even higher in heavily populated urban areas. Pediatricians, therefore, must understand its pathogenesis and management. Epidemiology Sixty percent of children who have atopic dermatitis manifest their disease in the first year of life; 90% do so by age 5 years. A genetically prone individual may not manifest the disease until exposed to a particular environmental situation. Onset has been associated with relocation from a rural to an urban location or from a region of high to low humidity. The course of adopic dermatitis is difficult to predict, although one 15-year longitudinal study revealed that the disease persisted in 60% of cases.

The skin is increasingly recognized as a component of the innate immune response, in addition to its role as a physical barrier. Although the deleterious effects of solar ultraviolet radiation (UVR), including immunosuppression and cutaneous tumorigenesis, are widely acknowledged, most studies to date have concentrated on adult skin. Despite the more sensitive nature of infant and toddler skin, little is known about its responses to UVR exposure, whether acute or long-term. Accumulating evidence suggests not only that the skin's barrier protection remains immature throughout at least the first 2 years of life but also that accumulation of UVR-induced changes in the skin may begin as early as the first summer of life. Such evidence not only affirms the importance of sun protection during the infant and toddler years but underscores the need for more research to establish evidence-based standards of care in this area. In this article we review recent studies in which differences between the skin properties of infants and young children and those of adults were compared, and we discuss the implications of these differences for sun-protection practices.

Background: Atopic dermatitis is an important cause of morbidity in children of all ages. Despite its high prevalence, there has been no examination of ways in which care for atopic dermatitis is delivered. Objective: This study reviewed the costs for care of childhood atopic dermatitis in an urban setting and estimated the national cost for treatment of the disease. Methods: We used data from one children's hospital to study the use of the emergency room for atopic dermatitis and used national data sets to estimate the cost of care in the United States. Results: A large proportion of visits occur in the emergency department, during daytime office hours, and mostly by patients who have public insurance. The total national cost for treatment of childhood atopic dermatitis is $364 million annually, which is a conservative estimate. Conclusion: Given its high prevalence, associated morbidity, and cost, resources must be better allocated to improve the organization of care for patients with atopic dermatitis.

We describe a 2½-year-old boy who has hirsutism and ringed creases of the extremities associated with an underlying smooth muscle hamartoma. Cutaneous findings in this child resemble those in other reports of the "Michelin tire syndrome." Histologic examination showed numerous well-demarcated fascicles of smooth muscle cells randomly distributed at all levels of the reticular dermis with haphazard orientation. These cells were immunoreactive with desmin, which confirmed their smooth muscle nature. In addition to the skin changes, this child has multiple unusual phenotypic anomalies, some of which have not previously been associated with the Michelin tire syndrome. These include distinctive facial dysmorphia, submucous cleft palate, lateral clefting of the mouth, genital, and dental anomalies. He also developed seizures at age 2½ years and has moderate developmental delay. The patient and his mother have apparently identical paracentric inversions of the long arm of chromosome 7 (46,XY,inv(7)(q22q31.3) with no detectable loss or gain of either chromosomal material or DNA markers from the cystic fibrosis (CFTR) region. The relevance, if any, of the karyotype abnormality to the phenotype in this child is discussed.

Background: A neonate with epidermolysis bullosa herpetiformis (EBH) (Dowling-Meara) had an undescribed ultrastructural and imrnunohistocnemical abnormality. Objective: The objective was to clarify the ultrastructural and immunohistochernical abnormalities in EBH to gain further insight into the pathogenesis of this disorder. Methods: Tissue from the patient was studied with routine histochemistry, electron microscopy, and immunohistochemistry. Results: Excessive clumping of tonofilaments on electron microscopic examination, anomalous hemidesmosomes, and immunohistochemical evidence of aberrant keratin expression by basal epidermal cells was found. Conclusion: This case of EBH provides further evidence for primary abnormalities involving cytoskeletal-membrane attachment plaque formation in this rare disorder.

Acropapular dermatitis of childhood is a symmetric self-limited papulovesicular exanthem that classically occurs on the cheeks, extensor extremities, and buttocks in young children. The eruption of acropapular dermatitis of childhood represents a reaction to a variety of infections usually of viral origin. We present a child with typical findings of acropapular dermatitis of childhood whose serologic workup revealed an acute Lyme infection.

This article discusses the evaluation and therapy for four difficult dermatologic problems: port wine stains and other flat vascular lesions, anogenital warts, congenital pigmented nevi, and ash-leaf macules.

Children with atopic dermatitis are more frequently colonized with Staphylococcus aureus than children without atopic dermatitis. However, little epidemiological data exist regarding the prevalence of methicillin-resistant S. aureus among children with atopic dermatitis. Recent studies have revealed an increasing prevalence of community-associated methicillin-resistant S. aureus among patients presenting to hospitals with serious bacterial infections, particularly those with cutaneous and soft tissue infections. As many atopic dermatitis patients are treated empirically with antibiotics for secondary skin infections, an understanding of the epidemiology of bacterial colonization and superinfection is essential for directing proper treatment in the atopic patient population. This study investigates the prevalence of risk factors for community-associated, methicillin-resistant S. aureus colonization among pediatric atopic dermatitis patients encountered at an academic pediatric dermatology clinic. An observational cross-sectional study was conducted at the Children's Hospital of Philadelphia in which 54 patients previously diagnosed with atopic dermatitis were enrolled. A detailed patient questionnaire, a complete cutaneous examination, and an evaluation of eczema severity according to the Eczema Area and Severity Index were completed at the time of enrollment. Bacterial cultures from the skin and nares were obtained to determine the frequency of colonization with either methicillin-sensitive S. aureus or methicillin-resistant S. aureus. Although most atopic dermatitis patients studied were colonized with S. aureus (43/54 [80%]), methicillin-resistant S. aureus was isolated from only seven atopic dermatitis patients (7/43 [16%]). Patients colonized with S. aureus were more likely to be male, to have been previously hospitalized, to have used a topical calcineurin inhibitor in combination with a topical steroid, and less likely to have used topical antibiotics. Bivariable analysis, however, revealed that only previous hospitalization was independently associated with an increased risk of methicillin-resistant S. aureus colonization. We observed that 80% of atopic dermatitis patients were colonized with S. aureus, and that of these patients, 16% of colonized patients were colonized with a methicillin-resistant strain. Methicillin-resistant S. aureus colonization was found to be significantly associated with previous hospitalization. Evidence also indicates that topical calcineurin inhibitors used in conjunction with topical steroids is associated with increased S. aureus colonization, while topical antibiotic use appears to decrease S. aureus colonization.

Epidermal nevi are benign congenital lesions that follow Blaschko lines. Epidermal nevi can demonstrate a variety of histologic patterns and contain hamartomatous hyperplasia of any epidermal or adnexal structure. We present the first reported instance of acne arising strictly within a preexisting keratinocytic epidermal nevus during puberty, which is a demonstration of genetic mosaicism in pilosebaceous units.

Recent trends indicate an increasing incidence of community-acquired methicillin-resistant Staphylococcus aureus (CAMRSA) in the general population, which is especially worrisome for clinicians caring for patients with atopic dermatitis (AD). Patients with AD are heavily colonized with S aureus and have impaired skin integrity and abnormal immune responses, which predisposes them to more invasive cutaneous infections (eg, cellulitis, furuncles, abscesses). In this report, we describe a child with severe AD who presented with CAMRSA skin abscesses. The presence of an atypical skin infection in patients with AD, particularly those unresponsive to conventional penicillinase-resistant penicillins and cephalosporins, should alert the clinician to the possibility of MRSA as the underlying etiology, and intervention should be directed accordingly.

Background Ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC) syndrome is a rare autosomal dominant disorder caused by mutations in the sterile α motif region of TP63 , a homologue of the tumor suppressor TP53 . Recent structure-function studies have identified complexities in the genotype-phenotype correlation of the p63 syndromes. Observations We report 2 sporadic cases of AEC syndrome in infants. Both patients demonstrated skin erosions with prominent scalp involvement. Histologic studies demonstrated mild basal layer vacuolization and rare dyskeratotic keratinocytes, with evidence of both acantholysis and cytolysis at the blister edge. Immunohistochemistry using anti-p63 monoclonal antibody demonstrated basal epidermal nuclear staining in both healthy control and patient tissue samples. Ultrastructural studies showed focal disruption of anchoring fibrils near the blister edge of one patient and normal desmosomes, hemidesmosomes, and basement membrane zone in the nonblistered skin of the other patient. The DNA analysis of each patient revealed 2 novel missense mutations in the TP63 gene that resulted in L514S and R555P amino acid substitutions within the sterile α motif region of the p63 protein. Conclusions We report 2 novel TP63 mutations resulting in AEC syndrome. The R555P mutation is the most carboxy-terminal of all the reported AEC missense mutations of p63. The presence of skin fragility, manifested as erosive skin lesions in body areas in addition to the scalp, is postulated to be an important diagnostic feature of AEC syndrome.

A full-term 1-day-old boy weighing 3140 g was transferred to our tertiary care children’s hospital with large areas of skin defects on his scalp, trunk, and extremities. His parents were nonconsanguineous, and the results of prenatal maternal laboratory screening for human immunodeficiency virus, hepatitis B, group B streptococci, and rapid plasma reagin were negative. Intrauterine fetal death of a twin had been reported at 16 to 18 weeks of gestation. No other complications were noted during the pregnancy. On physical examination, a full-thickness ulcer of the scalp vertex was observed extending from the anterior fontanel to the posterior fontanel in an area overlying the sagittal sinus. The defect measured 5 7 cm and revealed exposed dura and subcutaneous vessels (Figure 1). Two symmetric, vertically oriented, full-thickness defects, each measuring 3.2 cm, with minimal surrounding erythema, were noted on the lateral aspect of the abdomen (Figure 2). Examination of the knees revealed symmetric full-thickness defects that appeared as yellow-red, indurated ulcers. Symmetric, linear scars were observed bilaterally in the axillary and inguinal creases. Magnetic resonance imaging of the head and a magnetic resonance venogram revealed patent major intracranial venous sinuses and slight hypoplasia of the left transverse sinus (considered a normal congenital variation). A relative lack of subcutaneous fat over the superior aspect of the skull was also observed, consistent with the clinical presentation. Concern that the extensive open defect posed a high risk for sagittal sinus thrombosis, hemorrhage, or meningitis prompted plastic surgery and neurosurgery consultations. The scalp defect was repaired by plastic surgery with a skin graft, while the remaining areas of aplasia cutis congenita were allowed to heal by secondary intention. Over the next 3 weeks, the scalp healed well, without complications, as did the other areas of aplasia on the abdomen and knees. DIAGNOSTIC CHALLENGE

A 14-month-old girl and a 7-month-old boy each presented with a diffuse dermatitis, whole body edema, and hypoalbuminemia. The diets of both infants consisted almost entirely of Rice Dream, a rice-based, protein-poor beverage. Both infants were diagnosed with kwashiorkor, which resolved with protein supplementation. Clues from the physical examination, a diet history, appropriate laboratory examinations, and an index of suspicion are crucial in promptly diagnosing and treating infants with kwashiorkor. Manufacturers of rice beverages should appropriately warn parents about the dangers of using their products as infant nourishment.

The clinical presentation of tinea capitis may be varied and confusing. This results in delays in diagnosis, loss to follow-up, and spread of the infection. This article describes the use of calcofluor white (CFW) as a method for rapid diagnosis of tinea capitis. One hundred children with scaling scalps were evaluated. Specimens were obtained for fungal culture from each patient. Scales and hair were tested using the CFW test. The CFW test was positive in 58 (76%) of those with positive fungal cultures and 10 (42%) with negative fungal cultures. The sensitivity and specificity of the test were 76 and 58%, respectively. The relative ease and rapidity of the CFW test allows for rapid diagnosis of tinea capitis, immediate treatment of patients, and decreased loss to follow-up.

Necrolytic acral erythema is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma, and nutritional deficiency syndromes. Necrolytic acral erythema is distinguished by its association with hepatitis C infection and its predominantly acral distribution. We describe a pediatric patient with necrolytic acral erythema whose eruption resolved with hyperalimentation and combination interferon and ribavirin therapy, despite the persistence of detectable viral load and continued hepatic and renal insufficiency.

Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.

Group A β-hemolytic streptococci have been implicated in a variety of common childhood cutaneous infections. Infants and young children may be particularly susceptible to a form of streptococcal intertrigo that has heretofore been underrecognized in this population. Manifesting as intense, fiery-red erythema and maceration in the intertriginous folds of the neck, axillae, or inguinal spaces, the condition is characterized by a distinctive foul odor and an absence of satellite lesions. Specific clinical features help differentiate this condition from its clinical mimics. Topical and oral antibiotic therapy with or without concomitant low-potency topical steroid application is generally curative.

Therapy for kerions was evaluated by randomly assigning 30 patients to one of four treatment groups: group A griseofulvin, group B griseofulvin plus erythromycin, group C griseofulvin plus prednisone, and group D griseofulvin, erythromycin, and prednisone. Data indicate that antibiotic and steroid therapy, in addition to griseofulvin, may reduce scaling and pruritus, but does not reduce the time it takes for kerions to flatten.

Background Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are benign lymphocytic infiltrates of the skin that classically present as either a recurrent papulonecrotic eruption (PLEVA) or a persistent, scaling, papular eruption (PLC). Observations of both types of lesions present on individual patients have led to speculation that both entities are related. Previous studies evaluating the DNA of biopsy specimens from patients with PLEVA and PLC revealed clonal T-cell receptor β gene rearrangements. Objective To analyze and compare the T-cell populations between lesions of PLEVA and PLC. Design Retrospective and prospective analysis of patient tissue samples, classified by histologic analysis. Extracted DNA from 13 skin biopsy specimens with the diagnosis of PLC and 14 skin biopsy specimens with the diagnosis of PLEVA was analyzed by polymerase chain reaction/denaturing gradient gel electrophoresis (PCR/DGGE). Setting Molecular diagnostic laboratory at an academic medical center. Patients Twenty-seven tissue samples were obtained from patients with a histologic diagnosis of PLEVA or PLC. These samples were analyzed by PCR/DGGE. Main Outcome Measure The presence or absence of T-cell receptor gene rearrangements on PCR/DGGE analysis corresponding to a clonal population of T cells. Results Of 14 PLEVA specimens, 8 (57%) demonstrated monoclonal T-cell receptor gene rearrangements; 1 (8%) of 13 PLC specimens showed a gene rearrangement ( P = .008, Fisher exact test). Conclusions Our results demonstrate the polyclonal nature of the lymphocytic infiltrate found in almost all of the PLC specimens, which contrasts with the monoclonal nature found in most of the PLEVA specimens. These differences may represent different stages of the clinical evolution of a single entity that results from varying host immune responses to pathogenic factors. Specifically, we propose that PLEVA is a benign clonal T-cell disorder in which the clone arises from a subset of T cells in lesions of PLC. The host immune response to this clone determines the clinical and histologic findings in PLEVA.

The pediatric patient may present with a variety of dermatoses that either involve the eye primarily or are secondary to a more generalized process. The oculoplastic surgeon should become familiar with disorders occurring around the eye that are benign or may herald an underlying dermatologic disorder.

Types of lesions and patterns of distribution aid in the recognition of viral exanthems. Varicella and hand-foot-and-mouth disease can appear atypically in areas of inflammation. We describe 5 cases of morphologically altered exanthems that localized early and preferentially to areas of inflammation. (J Am Acad Dermatol 1999;40:345-6.)

Staphylococcus aureus colonization is common in atopic dermatitis (AD) and can exacerbate the disease. Additionally, some evidence shows that patients with AD may act as reservoirs for S. aureus transmission to others. This study compared S. aureus colonization in AD patients and their caregivers with control patients and their caregivers. Quantitative cultures were obtained from the lesions, clinically normal skin, hands, and anterior nares of 100 patients with AD, 100 controls with other cutaneous disorders, and 200 caregivers. AD patients had a significantly greater carriage of S. aureus from lesional and clinically normal skin as well as the hand. Significant increases in carriage of S. aureus were found in the anterior nares and hands of caregivers of AD patients compared with control caregivers. Topical corticosteroid use did not affect recovery of S. aureus. There was a significant correlation between recovery of S. aureus from lesional skin and recovery from the anterior nares (p = .002) and hands (p < .0001). These findings suggest that the anterior nares and the hands may be important reservoirs and vectors for transmission of S. aureus to lesional skin and to close contacts of these patients.

Pyoderma gangrenosum (PG) may occur as a purely cutaneous disorder, but is usually seen in adults with inflammatory bowel disease 1-3 or malignancy.a, 5 Although PG has been described in children with no underlying systemic disease, 6, 7 it has never been reported in association with Iracheitis. We describe a 9-month-old boy with PG in whom respiratory failure developed as a result of a noninfectious necrotizing tracheitis.

Quanlitative and semiquantitative bacterial culture specimens were obtained from 44 patients with kerions. Staphylococcus aureus was isolated from the scalp surface overlying the kerion in 29% of patients and from the pus within the kerion in 48% of patients. Gram-negative bacteria were found in the same locations in 11.3% and 18% of patients, respectively. The data indicate that bacteria are frequently cultured from kerions.

Background Impaired skin integrity in patients with Netherton syndrome (NS) results in significant systemic absorption of topically applied medications. Some have advocated the administration of pimecrolimus, 1%, topical cream for the treatment of patients with NS. Insufficient data exist with regard to its safety, systemic absorption, and efficacy. Observations An exploratory study was conducted involving 3 children with NS who received twice-daily application of pimecrolimus, 1%, cream over 18 months. There were no notable abnormalities in hematologic or chemistry profiles. Blood levels of pimecrolimus ranged from 0.625 to 7.08 ng/mL, with peak levels reached during the first month in all 3 patients. Dramatic reductions were observed in the Netherton Area and Severity Assessment, Eczema Area and Severity Index, Investigator Global Evaluation of Disease, and pruritus scores compared with baseline levels. Conclusions Use of pimecrolimus, 1%, cream was well tolerated and demonstrated marked improvements in nearly all of the parameters evaluated. Patients treated with pimecrolimus responded rapidly, within the first month of treatment, and improvement persisted throughout the study period. In adult patients receiving oral pimecrolimus, blood levels as high as 54 ng/mL for 3 months have not shown clinically significant immunosuppression. Absorption of pimecrolimus, 1%, cream was detectable, but levels were much lower than expected even when applied to 50% of total body surface area. Larger studies are warranted to determine the safety and efficacy of pimecrolimus, 1%, cream in the treatment of NS. Trial Registration clinicaltrials.gov Identifier:NCT00208026

Cutaneous fragility at age 2 years with blistering, scarring, milia, and hypertrichosis at age 4 years were noted in an otherwise healthy girl who had no family history of porphyria. Results of porphyrin analyses of urine, serum, and red blood cells revealed a pattern consistent with porphyria cutanea tarda. Red blood cell uroporphyrinogen decarboxylase activity was diminished to approximately 50% of normal in the child and in her mother and maternal grandmother, who were without symptoms; activity was normal in her sister, father, and maternal grandfather. Therapeutic phlebotomies were followed by a biochemical and clinical remission.

Many conditions present in childhood with vesicles and bullae, ranging from benign conditions to life-threatening diseases. This article reviews selected blistering disorders, with emphasis on new information that is helpful to the practicing physician. Entities that are discussed in this article include childhood zoster and fetal varicella syndrome, urticaria pigmentosa, scabies, bullous bug bites, lichen sclerosus et atrophicus, epidermolysis bullosa, and erythema multiforme.